Granulomatosis with Polyangiitis (Wegener’s Granulomatosis) Co-Existing with Antiphospholipid Syndrome – An Unusual Cutaneous Presentation

• Published in: Pakistan journal of medicine and dentistry 2024-08, Vol.13 (3), p.183-187
• Main Authors: Neha Rana, Humaira Talat, Maryam Rehan, Reema Mirza, Batool ur Rehman
• Format: Article
• Language: English
• ISSN: 2313-7371; 2308-2593
• DOI: 10.36283/PJMD13-3/026

Granulomatosis with polyangiitis (GPA) is an autoimmune disease that causes inflammation of the small blood vessels and involves many organs, most commonly the upper and lower respiratory tracts and the kidneys. GPA can also affect other organs, such as the eyes, skin, and nervous system. Meanwhile, Antiphospholipid syndrome (APS) is known to cause arterial or venous thrombosis in various organ systems. We report the case of a 54-year-old Pakistani man diagnosed with granulomatosis with polyangiitis co-existing with antiphospholipid syndrome. He presented with unusual cutaneous manifestations, positive c-ANCA, antiphospholipid antibodies, and distinctive renal histopathological features that helped rule out other potential differentials. The patient ultimately received treatment with a high dose of prednisolone, monthly pulses of cyclophosphamide, and anticoagulation, leading to significant improvement in both clinical and laboratory findings. This case underscores the complexity of autoimmune diseases and the importance of comprehensive evaluation for accurate diagnosis and management. Keywords: Gangrene, Wegener’s Granulomatosis, Antiphospholipid Syndrome, Anti Neutrophil Cytoplasmic Antibodies, Antiphospholipid Antibodies.